Explanipedia

Prospective Multicenter Longitudinal Measurement of Liver Stiffness in School-Age Children With Cholestatic Liver Disease Open

Benjamin L. Shneider, Nathan P. Goodrich, Wen Ye, Jean P. Molleston, Daniel H. Leung , et al. · 2025

Surprisingly, longitudinal measurement of LSM and laboratory parameters of liver disease severity over 2 years in school-age children with compensated BA, a1-AT, and ALGS did not reveal significant change, consistent with slow progression …

Sarcopenia is associated with osteopenia and impaired quality of life in children with genetic intrahepatic cholestatic liver disease Open

Julia M. Boster, Nathan P. Goodrich, Cathie Spino, Kathleen M. Loomes, Estella M. Alonso , et al. · 2023

Background: Sarcopenia occurs in pediatric chronic liver disease, although the prevalence and contributing factors in genetic intrahepatic cholestasis are not well-described. The objective of this study was to measure muscle mass in school…

Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease Open

Daniel H. Leung, Sridevi Devaraj, Nathan P. Goodrich, Xinpu Chen, Deepthi Rajapakshe , et al. · 2022

Background and Aims: Detailed investigation of the biological pathways leading to hepatic fibrosis and identification of liver fibrosis biomarkers may facilitate early interventions for pediatric cholestasis. Approach and Results: A target…

Use of funded multicenter prospective longitudinal databases to inform clinical trials in rare diseases—Examination of cholestatic liver disease in Alagille syndrome Open

Benjamin L. Shneider, Binita M. Kamath, John C. Magee, Nathan P. Goodrich, Kathleen M. Loomes , et al. · 2022

The conduct of long‐term conventional randomized clinical trials in rare diseases is very difficult, making evidenced‐based drug development problematic. As a result, real‐world data/evidence are being used more frequently to assess new th…

Mutation Analysis and Disease Features at Presentation in a Multi‐Center Cohort of Children With Monogenic Cholestasis Open

Paula M. Hertel, Laura N. Bull, Richard J. Thompson, Nathan P. Goodrich, Wen Ye , et al. · 2021

Objectives: To advance our understanding of monogenic forms of intrahepatic cholestasis. Methods: Analyses included participants with pathogenic biallelic mutations in adenosine triphosphate (ATP)‐binding cassette subfamily B member 11 ( A…

Functional Elements Associated with Hepatic Regeneration in Living Donors after Right Hepatic Lobectomy, Open

Gregory T. Everson, John C. Hoefs, Claus U. Niemann, Robert E. Dupuis, Shannon Lauriski , et al. · 2020

We quantified rates of hepatic regeneration and functional recovery for 6 months after right hepatic lobectomy in living donors for liver transplantation.

Nonfasted Liver Stiffness Correlates with Liver Disease Parameters and Portal Hypertension in Pediatric Cholestatic Liver Disease Open

Benjamin L. Shneider, Nathan P. Goodrich, Wen Ye, Cindy Sawyers, Jean P. Molleston , et al. · 2020

Elastographic measurement of liver stiffness is of growing importance in the assessment of liver disease. Pediatric experiences with this technique are primarily single center and limited in scope. The Childhood Liver Disease Research Netw…

Outcomes of Childhood Cholestasis in Alagille Syndrome: Results of a Multicenter Observational Study Open

Binita M. Kamath, Wen Ye, Nathan P. Goodrich, Kathleen M. Loomes, René Romero , et al. · 2020

Alagille syndrome (ALGS) is an autosomal dominant multisystem disorder with cholestasis as a defining clinical feature. We sought to characterize hepatic outcomes in a molecularly defined cohort of children with ALGS‐related cholestasis. T…

Best practices to optimize utilization of the National Living Donor Assistance Center for the financial assistance of living organ donors Open

Amit K. Mathur, Zoe A. Stewart Lewis, Patricia H. Warren, Marie-Claire Walters, Kimberly A. Gifford , et al. · 2019

Living organ donors face direct costs when donating an organ, including transportation, lodging, meals, and lost wages. For those most in need, the National Living Donor Assistance Center (NLDAC) provides reimbursement to defray travel and…

Clinically Evident Portal Hypertension Open

Lee M. Bass, Benjamin L. Shneider, Lisa Henn, Nathan P. Goodrich, John C. Magee · 2019

Portal hypertension (PHT) is a major cause of morbidity and mortality in pediatric liver diseases. Thus, research into causes and disease modifiers in PHT in these conditions is vitally important. PHT is rarely directly or indirectly measu…

Graphical representation of survival curves in the presence of time-dependent categorical covariates with application to liver transplantation Open

Abigail R. Smith, Nathan P. Goodrich, Charlotte A. Beil, Qian Liu, Robert M. Merion , et al. · 2018

Graphical representation of survival curves is often used to illustrate associations between exposures and time-to-event outcomes. However, when exposures are time-dependent, calculation of survival probabilities is not straightforward. Ou…

Bone Density in Children With Chronic Liver Disease Correlates With Growth and Cholestasis Open

Kathleen M. Loomes, Cathie Spino, Nathan P. Goodrich, Thomas N. Hangartner, Amanda E. Marker , et al. · 2018

Osteopenia and bone fractures are significant causes of morbidity in children with cholestatic liver disease. Dual‐energy X‐ray absorptiometry (DXA) analysis was performed in children with intrahepatic cholestatic diseases who were enrolle…

Kidney transplant graft outcomes in 379 257 recipients on 3 continents Open

Robert M. Merion, Nathan P. Goodrich, Rachel Johnson, Stephen P. McDonald, Graeme R. Russ , et al. · 2018

Estimating the effect of a rare time‐dependent treatment on the recurrent event rate Open

Abigail R. Smith, Danting Zhu, Nathan P. Goodrich, Robert M. Merion, Douglas E. Schaubel · 2018

In many observational studies, the objective is to estimate the effect of treatment or state‐change on the recurrent event rate. If treatment is assigned after the start of follow‐up, traditional methods (eg, adjustment for baseline‐only c…

Graphical representation of survival curves in the presence of time-dependent categorical covariates with application to liver transplantation Open

Abigail R. Smith, Nathan P. Goodrich, Charlotte A. Beil, Qian Liu, Robert M. Merion , et al. · 2018

Graphical representation of survival curves is often used to illustrate associations between exposures and time-to-event outcomes. However, when exposures are time-dependent, calculation of survival probabilities is not straightforward. Ou…

Biliary reconstructive techniques and associated anatomic variants in adult living donor liver transplantations: The adult‐to‐adult living donor liver transplantation cohort study experience Open

Talia Baker, Michael A. Zimmerman, Nathan P. Goodrich, Benjamin Samstein, Elizabeth A. Pomfret , et al. · 2017

Living donor liver transplantation (LDLT) is a technically demanding endeavor, requiring command of the complex anatomy of partial liver grafts. We examined the influence of anatomic variation and reconstruction techniques on surgical outc…

National assessment of early hospitalization after liver transplantation: Risk factors and association with patient survival Open

Pratima Sharma, Nathan P. Goodrich, Douglas E. Schaubel, Abigail R. Smith, Robert M. Merion · 2017

Hospitalization is known to occur frequently in the first 6 months following liver transplantation (LT). Using a novel data linkage between the Scientific Registry of Transplant Recipients and Centers for Medicare and Medicaid Services, ou…

Hepatic Hemodynamics and Portal Flow Modulation Open

Jean C. Emond, Nathan P. Goodrich, James J. Pomposelli, Talia Baker, Abhinav Humar , et al. · 2017

These results suggest the need for a study using a prespecified portal flow modulation protocol with defined indications to better define the effects of these interventions.

Issue Information ‐ TOC Open

Garrett R. Roll, Peter G. Stock, Bruno Roche, Didier Samuel, Juan J. Araiz , et al. · 2016

Acute liver failure due to secondary hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition and is considered by many as a contraindication for liver transplantation.The diagnosis is challenging and based on the …

National Assessment of Hospitalization Rates for Incident End-Stage Renal Disease After Liver Transplantation Open

Nathan P. Goodrich, Douglas E. Schaubel, Abigail R. Smith, Robert M. Merion, Pratima Sharma · 2016

Hospitalization rates increased significantly for LT recipients after ESRD onset. Early risk factor modification efforts targeting patients who are at high ESRD risk may reduce post-LT ESRD incidence and hence decrease morbidity and cost a…

Recurrent primary sclerosing cholangitis in the Adult‐to‐Adult Living Donor Liver Transplantation Cohort Study: Comparison of risk factors between living and deceased donor recipients Open

Fredric D. Gordon, David S. Goldberg, Nathan P. Goodrich, Anna S. Lok, Elizabeth C. Verna , et al. · 2016

Primary sclerosing cholangitis (PSC) recurs in 15%‐25% of patients transplanted for PSC. In the United States, PSC transplant patients are more likely to receive an organ from a living donor (LD) than patients without PSC. Our aims were to…

Patterns of Early Allograft Dysfunction in Adult Live Donor Liver Transplantation Open

James J. Pomposelli, Nathan P. Goodrich, Jean C. Emond, Abhinav Humar, Talia Baker , et al. · 2016

Early allograft dysfunction can be defined using postoperative day 7 laboratory values that are highly predictive of early graft failure within 90 days. Risk factors associated with EAD after LDLT include: graft type and size, preoperative…

Decision support for organ offers in liver transplantation Open

Michael L. Volk, Nathan P. Goodrich, Jennifer C. Lai, Christopher J. Sonnenday, Kerby Shedden · 2015

Organ offers in liver transplantation are high‐risk medical decisions with a low certainty of whether a better liver offer will come along before death. We hypothesized that decision support could improve the decision to accept or decline.…

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